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囊性纤维化的外科考虑因素:普通外科医生需要了解的知识。

Surgical considerations in cystic fibrosis: what every general surgeon needs to know.

机构信息

AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.

Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.

出版信息

ANZ J Surg. 2022 Oct;92(10):2425-2432. doi: 10.1111/ans.17948. Epub 2022 Aug 3.

DOI:10.1111/ans.17948
PMID:35920692
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9804981/
Abstract

Cystic fibrosis (CF) is a complex multiorgan disease, which often affects the gastrointestinal tract. With improved CF specific therapies and multidisciplinary management, patients with CF are now living longer with a median life expectancy of around 50 years. This increased life expectancy has resulted in corresponding increase in presentations of the CF patient with comorbid surgical conditions that were never important considerations. Investigations and management of these conditions, such as distal intestinal obstruction syndrome and colorectal cancer warrant good clinical understanding of the unique challenges that CF patients present including chronic immunosuppression, impaired respiratory function and their multi-organ dysfunction. The purpose of this review is to provide general surgeons with a contemporary update on the CF related surgical issues as they are likely to become increasingly involved in the care of these complex patients and form an integral part of the multidisciplinary team.

摘要

囊性纤维化(CF)是一种复杂的多器官疾病,常影响胃肠道。随着 CF 特异性治疗和多学科管理的改进,CF 患者的寿命现在更长,中位预期寿命约为 50 年。这种预期寿命的延长导致伴有合并症的 CF 患者就诊数量相应增加,这些合并症以前从未是重要的考虑因素。对于这些疾病的检查和管理,例如远端肠梗阻综合征和结直肠癌,需要很好地了解 CF 患者的独特挑战,包括慢性免疫抑制、呼吸功能受损和多器官功能障碍。本综述的目的是为普通外科医生提供有关 CF 相关手术问题的最新信息,因为他们可能会越来越多地参与这些复杂患者的治疗,并成为多学科团队的重要组成部分。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b91/9804981/00b6d753bb41/ANS-92-2425-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b91/9804981/c4e5af753283/ANS-92-2425-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b91/9804981/feabc0966c3a/ANS-92-2425-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b91/9804981/00b6d753bb41/ANS-92-2425-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b91/9804981/c4e5af753283/ANS-92-2425-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b91/9804981/feabc0966c3a/ANS-92-2425-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b91/9804981/00b6d753bb41/ANS-92-2425-g001.jpg

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Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators.在囊性纤维化跨膜电导调节剂调节剂时代改变囊性纤维化胃肠道并发症治疗模式。
Paediatr Respir Rev. 2022 Jun;42:9-16. doi: 10.1016/j.prrv.2020.12.001. Epub 2020 Dec 24.
3
Evaluation of a five-year predicted survival model for cystic fibrosis in later time periods.
评估后期囊性纤维化五年预测生存率模型。
Sci Rep. 2020 Apr 20;10(1):6602. doi: 10.1038/s41598-020-63590-8.
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Pancreatic cystosis in cystic fibrosis: Sometimes a bike ride can help you decide.囊性纤维化中的胰腺囊肿病:有时候骑车能帮你做出决定。
Respir Med Case Rep. 2020 Feb 5;29:101018. doi: 10.1016/j.rmcr.2020.101018. eCollection 2020.
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Cystic fibrosis carriers are at increased risk for a wide range of cystic fibrosis-related conditions.囊性纤维化携带者患多种囊性纤维化相关疾病的风险增加。
Proc Natl Acad Sci U S A. 2020 Jan 21;117(3):1621-1627. doi: 10.1073/pnas.1914912117. Epub 2019 Dec 27.
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